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Abstract Background Umbilical endometriosis is a rare entity accounting for 0.5–4% of cases with endometriosis. Case presentation Here we report a rare case of umbilical endometriosis with concurrent ovarian endometriomas in a 37-year old primiparous Iranian woman. Conclusion This interesting coexistence reflects the importance of thorough gynecological assessment in patients with cutaneous endometriosis to enable appropriate management.